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Biomedical subjects

G V Watters

Publications and source records attributed to G V Watters.

At least 19 recordsLinked to original sources
PubMed1992

King syndrome: a genetically heterogenous phenotype due to congenital myopathies.

We report on a patient with myopathy, kyphoscoliosis, joint contractures, and a facial appearance consistent with King syndrome. Unlike other reported cases, our patient had hyperextensible joints, normal stature, and pectus excavatum. The cardiac ventricles, aorta, and pulmonary artery were dilated. Malignant hyperthermia did not occur under anaesthesia although there was a transient increase in CK levels. Muscle bulk and tone were significantly decreased but collagen and elastin fibres were normal. The variable clinical presentation of King syndrome suggests that the manifestations are caused by different congenital myopathies and in all cases there is probably an increased risk of malignant hyperthermia.

Child↗
PubMed1991

Syndrome of mental retardation and distal arthrogryposis in sibs.

Two sisters presented with a syndrome of characteristic facial anomalies and distal arthrogryposis. The older sister is now 4 years old and is severely mentally retarded. Her sister died of respiratory failure due to hypoplastic lungs shortly after birth. The occurrence of this potentially lethal syndrome in 2 sisters with unaffected parents suggests autosomal recessive inheritance.

Arthrogryposis↗
PubMed1990

The sequelae of Haemophilus influenzae meningitis in school-age children.

BACKGROUND: Previous data on the consequences of Haemophilus influenzae type b meningitis for school-age children have been inconsistent, and much of the information on risk factors has been inconclusive. The present study was designed to evaluate the sequelae of this disease with a protocol for the comprehensive assessment of neuropsychological function. METHODS: Ninety-seven school-age children (mean age, 9.6 years), each of whom had a school-age sibling, were recruited from a survey of the medical records of 519 children treated for H. influenzae type b meningitis between 1972 and 1984 (at a mean age of 17 months) at the children's hospitals of Toronto, Ottawa, and Montreal. Of the 97 children, 41 had had an acute neurologic complication. Sequelae were assessed by comparing the index children with their nearest siblings on the basis of standardized measures of cognitive, academic, and behavioral status. RESULTS: Only 14 children (14 percent) had persisting neurologic sequelae: sensorineural hearing loss in 11 (unilateral in 6 and bilateral in 5), seizure disorder in 2, and hemiplegia and mental retardation in 1. Although the total sample of index children scored slightly below the siblings in reading ability, the 56 children without acute-phase neurologic complications (58 percent) were indistinguishable from their siblings on all measures. The differences between the groups were small even for the 41 pairs in which the index child had had an acute neurologic complication (mean full-scale IQ, 102 for the index children vs. 109 for the siblings). Sequelae were also associated with lower socioeconomic status and a lower ratio of glucose in cerebrospinal fluid to that in blood at the time of the meningitis. Behavioral problems were more prominent in index boys than index girls and in those who were older at the time of testing, but sex and age were not related to cognitive or academic sequelae. CONCLUSIONS: We find a favorable prognosis for the majority of children who are treated for meningitis caused by H. influenzae type b.

Adolescent↗
PubMed1988

Encephalitis among Cree children in northern Quebec.

We report a neurological disease among Cree Indian children in a northern Quebec village. The disease manifests as severe mental retardation, cerebral atrophy with white matter changes and calcifications, and systemic immunological abnormalities. Eleven cases are known in five families. The familial incidence of cases and the high degree of parental consanguinity suggest a genetic contribution. We propose that this entity may be caused by an unusual viral infection in a genetically vulnerable host.

Adolescent↗
PubMed1988

Leukoencephalopathy among native Indian infants in northern Quebec and Manitoba.

We report 14 cases of a severe familial leukoencephalopathy among native North American Indian infants in northern Quebec and Manitoba. Affected infants have hypotonia and mild motor delay, followed by seizures, hypotonia or spasticity, eye deviation, and abnormal posture during a febrile illness around 6 months of age. Death follows a rigid, vegetative state that manifests days to months after disease onset and is marked in some cases by prominent autonomic disturbances, blindness, and cessation of head growth. Symmetrical hemispheric white matter lucencies and diffuse hypomyelination of the cerebral hemispheres and brainstem are the radiological and pathological hallmarks. This disease differs from the known diseases of cerebral myelin. An autosomal recessive pattern of inheritance awaits statistical confirmation. The proposed cause is a delay in development or abnormal turnover of central nervous system myelin.

Brain↗
PubMed1987

Subacute necrotizing encephalomyelopathy (Leigh disease): CT study.

Leigh disease, or subacute necrotizing encephalomyelopathy (SNE), is a familial, degenerative disorder characterized by lesions of the gray and white matter in the brain and spinal cord. Low attenuation in the putamina on computed tomography (CT) scans is considered to be characteristic of the disease. The authors used CT to study five patients, in three of whom the disease was confirmed histologically. In one of the patients with documented SNE, there was extensive gray matter lucency with normal basal ganglia. CT scans obtained in a second patient showed diffuse, diseased white matter with focal cortical extension and bilateral caudate involvement. In the other three cases, CT scans revealed the usual changes that occur in the basal ganglia. The appearance of SNE on CT scans thus reflects the variable and widespread distribution of the disease. The absence of radiologically detectable abnormalities in the basal ganglia should not deter one from the diagnosis of Leigh disease given in the appropriate clinical context.

Basal Ganglia↗
PubMed1986

X-linked hydrocephalus.

Two French-Canadian families with seven cases of hydrocephalus in two generations are presented. The pattern of inheritance is consistent with an X-linked recessive transmission. The clinical and pathologic characteristics of this entity are reviewed. The anomaly of adducted thumbs was present in one patient and its cause is considered. The hypothesis of primary hydrocephalus and secondary compression of the aqueduct as the mechanism for aqueductal stenosis is discussed.

Brain↗
PubMed1985

Development of speech and language following bilateral frontal lesions.

Language and speech were studied in a young child with perinatally acquired bifrontal lesions. Bilateral frontal pathology seriously interfered with the development of intelligible speech and resulted in a persistent expressive aphasia. Analysis of the neuropsychological profile indicated impairments in intelligence and language comprehension. These deficits, however, were considered secondary to the profound speech programming disorder. The findings indicate that, despite the plasticity of the immature central nervous system, bilateral frontal injury sustained at an early age precludes the development of intelligible speech. Furthermore, structurally intact cortical regions outside the territories of the speech zones fail to mediate normal speech and language development.

Aphasia↗
PubMed1985

Aphasia and handedness in relation to hemispheric side, age at injury and severity of cerebral lesion during childhood.

The effects of the variables of hemispheric side of lesion, age at injury and severity of cerebral damage on language performance and hand dominance were investigated in groups of hemiparetic children. Severity of cerebral damage was defined by the degree of structural abnormality shown on computed tomography (CT) scans. Tests of auditory verbal comprehension and object naming were used as indicators of productive and receptive language skills. The responses to a series of questions on a handedness inventory provided a rated measure of hand dominance. The results indicated that language deficits characterize the performance of all patient groups with left cerebral injuries. Impairments are more profound, however, in the case of left hemisphere injuries acquired after the age of 5 years. In addition, prenatal and early postnatal left cerebral lesions consistently result in strong sinistrality. It is concluded that the crucial variable underlying the demonstration of language deficits and left hand dominance is not severity of lesion but age at injury and hemispheric side of lesion.

Adolescent↗
PubMed1984

Recurrent aseptic meningitis secondary to intracranial epidermoids.

Two patients with recurrent aseptic meningitis are reported. Intracranial epidermoid tumors were eventually found in both patients. CSF changes in our first case with an epidermoid tumor were similar to those reported in Mollaret's meningitis, with an initial polymorphonuclear pleocytosis early in each attack, followed by a lymphocytic predominance several days later. Large cells which proved to be macrophages were also seen in the CSF of this patient. Both patients had meningitis attacks for several years before the underlying epidermoid tumor was found. Mollaret's meningitis remains a diagnosis of exclusion. These patients should remain tumor suspects for many years and deserve careful and repeated investigation.

Brain Neoplasms↗
PubMed1983

EEG abnormalities and convulsions in juvenile diabetes mellitus.

The clinical and EEG findings were reviewed for 270 juvenile children from the Montreal Children's Hospital Diabetic Clinic in an attempt to correlate the EEG findings at the onset of diabetes mellitus with the future risk of having a convulsion with a hypoglycemic reaction. Compared to a non-diabetic control population, the number of epileptiform EEG abnormalities was significantly increased in the initial EEG of diabetic patients who later had a hypoglycemic convulsion. The initial epileptiform EEG did not help to differentiate those diabetics with recurrent hypoglycemic induced convulsions from diabetics who would have only a single convulsion.

Age Factors↗
PubMed1983

Growth and development of full-term nonasphyxiated small-for-gestational-age newborns: follow-up through adolescence.

Previous studies of growth and development in small-for-gestational-age (SGA) infants have been plagued by several methodologic defects, including a lack of control for socioeconomic status and parental height and an inability to distinguish among the effects of prematurity, neonatal asphyxia, and intrauterine growth retardation. An attempt has been made to overcome these defects in a study of 33 full-term, nonasphyxiated small-for-gestational-age neonates born between 1960 and 1966 and 33 matched control infants of normal birth weight. The infants were followed up and compared for physical growth and sexual, neurologic, and cognitive development at ages 13 to 19 years. Significant deficits in height, weight, and head circumference were found among the SGA cohort, even after statistical adjustment for differences in socioeconomic status and parental height. Sexual development and bone age were not delayed in the SGA group, however, indicating that the deficits in growth are permanent. On neurologic and cognitive testing, the SGA group had trends toward lower scores but scores were well within the normal range. It is concluded that full-term nonasphyxiated SGA infants have an impaired potential for physical growth, but a good prognosis for neurologic and cognitive development. Previous findings of more severe cognitive deficits are attributed to a failure to distinguish the effects of isolated intrauterine growth retardation from effects due to asphyxia.

Adolescent↗
PubMed1982

Electrophysiological investigation of the auditory system in Friedreich's ataxia.

Auditory brainstem responses (ABRs) and cortical auditory evoked responses (AERs) were studied in a series of 16 Friedreich's ataxia patients who varied in age, degree of clinical involvement and duration of the disorder. The ABRs were markedly abnormal in all but the youngest patient, and the abnormalities reflected the severity and duration of the disease. The latencies of the AERs were significantly longer in the Friedreich's ataxia patients compared to normal controls, suggesting cortical as well as peripheral involvement of the auditory system. These data are discussed in terms of the neuropathology of the disorder and the similarities with the other sensory systems in Friedreich's ataxia patients.

Adolescent↗
PubMed1982

Sodium valproate in the treatment of the intractable childhood epileptic.

Sixty-five children with seizure disorders, who had been treated with multiple anticonvulsants but were poorly controlled, were selected from the Montreal Children's Hospital Convulsive Disorder Clinic and Neurology Service and were treated with sodium valproate (valproate). All types of seizure disorders were included in the group. Rapid oral absorption of the drug lead to peak plasma levels in one to three hours (later peaks occurring if administered after meals). A mean plasma half-life of 12.8 hours was calculated. Correlation between oral dose and plasma levels was poor. The side effects which occurred in this study were trivial. Drug interactions occurred with phenobarbital, diphenylhydantoin and clonazepam. Sixty percent of patients had a greater than 50% reduction in seizure frequency with sodium valproate, but the best response was in generalized absence seizures. Since all types of seizures responded to some degree, a trial of sodium valproate is warranted in intractable seizure disorders of childhood regardless of classification.

Adolescent↗